McPherson E, Clemens M
University of Pittsburgh, Pennsylvania, USA.
Am J Med Genet. 1996 Mar 1;62(1):58-60. doi: 10.1002/(SICI)1096-8628(19960301)62:1<58::AID-AJMG12>3.0.CO;2-U.
A chromosomally normal brother and sister shared a lethal pattern of anomalies including bilateral cleft lip and palate, hypertelorism, flat facial profile, flat occiput, complex congenital heart defect, and malrotation of the intestine. The male was large for gestational age, while his sister was normally grown. The girl had bifid thumbs, but the boy had only minor hand anomalies. These findings are not consistent with any previously recognized syndrome and represent a new condition with probable autosomal recessive inheritance.
一对染色体正常的兄妹表现出相同的致命性异常模式,包括双侧唇腭裂、眼距过宽、面部轮廓扁平、枕部扁平、复杂的先天性心脏缺陷和肠旋转不良。该男性胎儿为大于胎龄儿,而他的妹妹生长正常。女孩有拇指裂,而男孩只有轻微的手部异常。这些发现与任何先前公认的综合征均不相符,代表了一种可能为常染色体隐性遗传的新病症。